Case Report Primary Small Cell Neuroendocrine Carcinoma of Paranasal Sinuses Maliha Khan, 1 Sobia Nizami, 2 Aibek E. Mirrakhimov, 1 Benjamin Maughan, 3 Justin A. Bishop, 3 and William H. Sharfman 3 1 Presence Saint Joseph Hospital, Department of Internal Medicine, 2900 N. Lake Shore Drive, Chicago, IL 60657, USA 2 Department of Medicine, Aga Khan University, Karachi, Sindh 74800, Pakistan 3 Sidney Kimmel Comprehensive Cancer Center, John Hopkins University, Baltimore, MD 21287, USA Correspondence should be addressed to Maliha Khan; doc.maliha@gmail.com Received 26 April 2014; Revised 3 June 2014; Accepted 3 June 2014; Published 25 June 2014 Academic Editor: David W. Eisele Copyright © 2014 Maliha Khan et al. his is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Small cell neuroendocrine carcinoma of the paranasal sinuses is an extremely rare and aggressive neoplasm. Despite aggressive management, the tumor carries a poor prognosis, with a high risk of local recurrence or distant metastases. he management strategy is based on that for pulmonary small cell cancer and includes platinum-based chemotherapy combined with radiotherapy. We are reporting a case of an 89-year-old female patient diagnosed with small cell carcinoma of right-sided ethmoid and sphenoid sinuses. he tumor was found to have invaded the right orbit and anterior cranial fossa. Metastases to cervical lymph nodes and bone were also found. Due to the extended stage and poor prognosis of the patient, the management plan is palliative chemoradiotherapy. 1. Introduction Small cell carcinoma (SCC) is a poorly diferentiated neu- roendocrine tumor that most commonly occurs in the lung [1]. Small cell neuroendocrine carcinoma (SNEC) of the paranasal sinuses is an extremely rare and aggressive tumor that demonstrates rapid expansion and early hematogenous spread [2]. Although the tumor is responsive to initial local therapy [1], it is associated with frequent local recurrence and new distant metastases, leading to poor prognosis [2]. Here we report the case of a patient diagnosed with small cell neuroendocrine carcinoma of the ethmoid/sphenoid sinuses with local intracranial extension, who was referred to our center for management. 2. Case History An 89-year-old female with a history of hypertension, hyper- cholesterolemia, glaucoma, and osteoarthritis presented with a history of headaches and facial pain over the right side of her face for one month. Other symptoms included decreased vision in the right eye, intermittent diplopia, diiculty in closing the right eye, and gait unsteadiness. She described having overall fatigue and lower back pain, worse on walking, for the past 3 months. here was no past history of cigarette smoking. Physical examination was positive for complete right eyelid closure, no overlying skin changes and no cervical lymphadenopathy. Brain CT and MRI scans revealed a large sinonasal mass involving the ethmoid/sphenoid sinuses and extending into the right orbit and anterior cranial fossa (Figures 1, 2, and 3). Fine-need aspiration of the nasal mass conirmed the tumor as small cell carcinoma (Figure 4(a)). A PET scan was done to look for distant disease as well as a possible lung primary, which showed multiple bone metastases but no lung mass. Immunohistochemical studies demonstrated tumor positivity for AE1/AE3 in a dot-like pattern (Figure 4(b)), synaptophysin (Figure 4(c)), and chro- mogranin and were negative for CD45, S100, and myogenin. he tumor was also positive for high-risk HPV by in situ hybridization (Figure 4(d)). he patient was diagnosed with extensive stage small cell carcinoma of the ethmoid/sphenoid sinuses. Due to disseminated disease, she was scheduled for palliative chemotherapy with 6 cycles of carboplatin and etoposide every 21 days [3]. Hindawi Publishing Corporation Case Reports in Medicine Volume 2014, Article ID 874719, 4 pages http://dx.doi.org/10.1155/2014/874719